Endocrine Flashcards

1
Q

pituitary apoplexy-what is it, presentations, and treatment

A

acute hemorrhage into the pituitary gland

  • associated with preexisting pituitary adenoma
  • severe headache, cranial nerve involvement (bitemporal hemianopsia, ophthalmoplegia), meningeal irritation
  • treat with glucocorticoid replacement (prevent life-threatening hypotention) or surgical decompression
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2
Q

Kallmann syndrome-what is it

A

absence of GnRH secretory neuronsin the hypothalamus due to defective migration form the olfactory placoid

-associated with mutatio nin the KAL-1 gene or fibroblast growth factor receptor-1gene

–> central hypogonadism and anosmia, delayed puberty

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3
Q

mutation in what gene is associated with medullary thyroid cancer

A

RET proto-oncogene

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4
Q

what are the 3 P’s of MEN1

A

parathyroid adenomas, pituitary tumors, and pancreatic endocrine tumors

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5
Q

how can patients still metabolize fructose when fructokinase is deficient

A

hexokinase takes over fructose metabolism

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6
Q

what can and can’t use ketones for energy

A

can: brain, kidneys, cardiac muslce and skeletal muscle

can’t: erythrocytes b/c lack mitochondria

ketones produced int eh liver

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7
Q

how can ethanol cause hypoglycemia

A

by inhibiting gluconeogensis

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8
Q

clinical manifestations of congenital hypothyroidism

A

normal at birth
-lethargy, enlarged fontanelle, protruding tongue, umbilical hernia, poor feeding, constipation, dry skin, jaundice

-6 P’s: pot-bellied, pale, puffy-faced, protruding umbilicus, protuberant tongue, poor brain development

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9
Q

growth hormone stimulate release of insulin-like growth factor-1 (IGF-1) from what organ?

A

liver

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10
Q

how come you don’t see high urinary excretion of ketones with type 2 diabetes

A

high total insulin levels suppress ketone formation

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11
Q

mechanism of beta-blockers for thyrotoxicosis (thyroid storm)

A

decreases peripheral conversion of T4 to T3 by inhibiting iodothyronine deiodinase

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12
Q

presentation of urea cycle disorders

A

vomiting, tachypnea, and confusion/coma secondary to hyperammonemia

most common disorder: ornithine transcarbamylase deficiency–> elevated urinary orotic acid

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13
Q

avidin in egg whites binds to ______

A

biotin (vitamin B6)–> deficiency in biotin leads to dermatitis, alopecia, changes in mental status, myalgias, anorexia, and lactic acidosis

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14
Q

essential amino acid in phenylketonuria

A

Tyrosine–> cannot be synthesized from phenylalnine due to deficiency of phenylalanine hydroxylase so can’t convert phenylalanine to tyrosine

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15
Q

most common complication of statin

A

myopathy

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16
Q

how can glucocorticoids lead to hyperglycemia

A

increase hepatic gluconeogenesis and glycogenesis along with peripheral antagonism of insulin

17
Q

mechanism of action of Finasteride

A

5-alpha-reductase inhibitor that suppresses periperal conversion of testosterone to dihydrotestosterone

used for treatment of benign prostatic hyperplasia and androgenetic alopecia (increased in 5-alpha-reductase is associated with androgenetic alopecia)

18
Q

3 primary effects of PTH

A
  • increase osteoclastic bone resorption–> increase calcium and phosphate release into the circulation
  • increase renal calcium reabsorption and reduces phosphate reabsorption (excrete phosphate but keep calcium)
  • increased formation of active vitamin D, which increases intestinal calcium and phosphate absorptioin
19
Q

what is neurophysin

A

carrier protein for oxytocin and vasopressin (ADH), hormones produced within the paraventricular and supraoptic nuclei, respectively, and released from the posterior pituitary

-point mutation in neurophysin II lead to hereditary hypothalamic diabetes insipidus

20
Q

how can estrogen increase total T4

A

estrogen raises circulating thyroxine-binding globulin –> reduce free T4 and free T3

–> increase in thyroid hormone production

21
Q

side effect of niacin

A

-decrease renal excretion of uric acid leads to acute gouty arthritis, flushing, hyperglycemia, and hepatotoxicity

use to decrease hepatic synthesis of triglycerides and VLDL and reduces clearance of HDL

22
Q

mechanism of action for meglitinides

A

short-acting glucose-lowering medication

close ATP-dependent K+channel in the pancreatic beta cell membrane, inducing depolarization and stimulating insulin release

23
Q

mechanism of action for SGLT2 inhibitors

A

oral anti-diabetic

  • decrease proximal tubular reabsorption of glucose to promote urinary glucose loss
  • should be avoided in those with moderate-to-severe renal impairment
24
Q

side effect of thioamides (propylthiouracil, methimazole)

A

skin rash, agranulocytosis (rare), aplastic anemia, hepatotoxicity

methimazole=teratogen

25
Q

2 signs of hypocalcemia

associated with hypoparathyroidism

A

Chvostek sign: facial muscle contraction elicited by tapping on the facial nerve anterior to the ear

trousseau sign: carpopedal spasm triggered by prolonged inflation of a blood pressure cuff around arm

26
Q

what organelle is found in all steroid-producing cells (eg, cells in the adrenals, gonads, and liver)

A

smooth ER

27
Q

mechanism of actions of sulfonylureas (eg, glyburide)

A

increase insulin secretion rate of residual pancreatic islet beta-cells and reduce blood glucose levels in patients with type 2 diabetes mellitus

–> increase circulating C-peptide

28
Q

mechanism action of Leuprolide

A

GnRH analog to suppress testosterone levels in treatment of prostate cancer

pulsatile fashion–> agonist properties

continuous fashion–> downregulation of GnRH receptors on gonadotropin cells in the pituitary gland

29
Q

what is Hartnup disease

A

autosomal recessive metabolic disorder due to inactivating mutations affecting the neutral amino acid transporter (esp. tryptophan), in small intestine and proximal tubule of kidney

–> need tryptophan to get niacin so patient has niacin deficiency

30
Q

which glucose transport proteins (GLUTs) is the only one that is responsive to insulin?

A

GLUT4 (skeletal muscle cells and adipocytes)

the other ones are insulin-independent

31
Q

complications of familial chylomicronemia syndrome?

A

recurrent episodes of acute pancreatitis, lipemia retinalis (milky-appearing retinal vasculature), and eruptive xanthomas

syndrome- auto recessive with lipoprotein lipase (LPL) deficiency so can’t clear dietary lipid loads (esp. chylomicrons)

32
Q

how does the calcium levels regulate the secretion of parathyroid hormone (PTH)

A

through calcium-sensing receptors= transmembrane G-protein-coupled (metabotropic) receptors that help to regulate the secretion of parathyroid hormone (PTH) in response to changes in circulating calcium levels

33
Q

histological finding of medullary thyroid cancer

A

nests or sheets of polygonal or spindle-shaped cells with extracellular amyloid deposits derived from calcitonin

34
Q

alpha-2 adrenergic receptors _____ insulin secretion, and beta-2 adrenergic receptors ________ insulin secretion

A

inhibit; stimulate

35
Q

what are the acute effects of corticosteroids on the white blood cell count

A

increased neutrophil count (“demargination” of neutrophils previously attached to vessel wall)

decreased lymphocyte, monocyte, basophil, and eosinophil counts

36
Q

characteristic of Klinefelter syndrome

A

primary hypogonadism (elevated FSH and LH, low testosterone); long lower extremities; small, firm testes; and azoospermia

37
Q

methimazole vs. propylthiouracil (PTU)

A

both are antithyroid drugs used to decrease thyroid hormone production

methimazole preferred due to hepatotoxicity of PTU
methimazole is teratogenic so PTU is preferred in the first trimester of pregnancy

38
Q

complications of subclinical hyperthyroidism

A

hypertension, accelerated bone resorption, and atrial fibrillation

can also be caused by medications like levothyroxine

39
Q

prolactin production is stimulated by

A

TRH

in patients with primary hypothyroidism, the increased production of TRH by the hypothalamus can lead to hyperprolactinemia